Cystatin-B; Stefin B; PME; CST6

For Research Use Only. Not for use in diagnostic procedures.

E Coli
Source: Human

>95%

Liquid

1 mg/ml (lot specific)

Store at -20 degree C for 12 months.
Centrifuge the vial prior to opening.

Small volumes of CSTB recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

CSTB; also known as Cystatin B is an anti-protease implicated in myoclonus epilepsy; a degenerative disease of the central nervous system. The cystatin superfamily encompasses proteins that contain multiple cystatin like sequences. Some of the members are active cysteine protease inhibitors; while others have lost or perhaps never acquired this inhibitory activity. This protein is able to form a dimer stabilized by noncovalent forces and is thought to play a role in protecting against the proteases leaking from lysosomes. In cells; CSTB is located in the lysosomes and the cytoplasm; but also in the nucleus.

SDS-PAGE

13 kDa

cystatin B

cystatin-B

Cystatin-B

CST6; STFB??[Similar Products]

The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG repeat from 2-3 copies to 30-78 copies. [provided by RefSeq, Jul 2016]

CSTB: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1). EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Belongs to the cystatin family.

Protein type: Inhibitor

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: cytoplasm; cytosol; extracellular region; extracellular space; nucleolus

Molecular Function: cysteine protease inhibitor activity; endopeptidase inhibitor activity; protease binding; RNA binding

Biological Process: negative regulation of peptidase activity; negative regulation of proteolysis; neutrophil degranulation

Disease: Myoclonic Epilepsy Of Unverricht And Lundborg

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